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Boards.CP.Heme > DLBCL > Flashcards

DLBCL Flashcards

1
Q

Hans Method - DLBCL subtypes:

-Step 3

A

MUM1

  • Positive, then Non-GBC (ABC)
  • Negative, then GBC
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2
Q

What is true about late (>5 years) PTLD?

A
  • EBV Negative

- More Aggressive

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3
Q

Posttransplant Lymphoproliferative DIsorder (PTLD):

  • When do they most often occur?
  • What are the “heralded” by?
A
  • <1 year post-transplant

- Heralded by elevated EBV-DNA

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4
Q

Lymphomatoid Granulomatosis most commonly affects which organs? (4)
-What is it associated with?

A

-Respiratory
-Brain
-Kidney
-Liver
Associated with EBV and Immunodeficiency

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5
Q

DLBCL clinical presentation.

A
  • Rapidly enlarging lymph node or extranodal site
  • Localized at presentation

*BM involvement uncommon (10%)

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6
Q

ALK+ DLBCL:

  • Neoplastic cells
  • Nodal or Extranodal
  • HIV or EBV association
A
  • Immunoblastic/Plasmablastic that express ALK
  • Nodal
  • NOT ass. w/ HIV or EBV
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7
Q

What are the most common lymphomas in HIV? (5)

A
  • Burkitt Lymphoma
  • DLBCL (CNS)
  • Primary Effusion Lymphoma
  • Plasmablastic Lymphoma
  • Hodgkin Lymphoma
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8
Q

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

-Small lymphocyte IHC (+2;-3)

A 
Positive:
-Mixture of CD4+ and CD8+
Negative:
-CD57
-CD21
-CD23
*NO T-cell rosettes or small B cells
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9
Q

Primary Mediastinal (Thymic) Large B-cell Lymphoma:

  • Age
  • Morphology
A
  • Adult Females (F:M;2:1)

- Sclerosing lymphoma with large B cells entrapped within bands of sclerosis

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10
Q 
Primary Mediastinal (Thymic) Large B-cell Lymphoma:
-IHC (+5;-3)
A 
Positive:
-CD19
-CD20
-CD30
-CD45
-CD79a
Negative:
-CD5
-CD10
-Surface Igs
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11
Q

Hans Method - DLBCL subtypes:

-Step 1

A

CD10

  • Positive (>30%), then GCB
  • Negative, step 2
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12
Q

Ki67 expression in DLBCL.

A

60-99%

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13
Q

Which type of DLBCL, Germinal Center (GCB) or Activated B-cell (ABC; nonGCB), has a better response to treatment?

A

Germinal Center (GCB) type

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14
Q

TCRBCL Bone Marrow involvement pattern.

A

Paratrabecular lymphoid aggregates

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15
Q

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

  • Age
  • Morphology
A

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

  • 40 y/o (mean; kids-elderly)
  • Diffuse proliferation of small lymphocytes and histiocytes with scattered large B cells
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16
Q 
Primary Mediastinal (Thymic) Large B-cell Lymphoma:
-Molecular/Cytogenetics
A

Alterations in the MAL gene
-Gains in 9p (location of JAK2)

*NO rearrangement of BCL2 or BCL6

17
Q

Rearrangement of BCL6 is more common in what type of DLBCL?

A

Non-Germinal Center (ABC) type

18
Q

CD5 expressing DLBCL must be distinguished from what?

A

Blastoid MCL (bcl-1+)

19
Q

What are the most common Primary Immunodeficiencies (PID) with Lymphoproliferative disorders (LPD)? (5)

A
  • Ataxia Telangiectasia
  • Wiskott Aldrich syndrome
  • Common Variable Immunodeficiency
  • X-linked Lymphoproliferative disorder (Duncan disease)
  • Nijmegan Chromosomal Breakage Syndrome
20
Q

What gene rearrangements are present in 20-30% of DLBCL?

A

BCL2 and BCL6

21
Q

Hans Method - DLBCL subtypes:

-Step 2

A

BCL6

  • Negative, then Non-GBC (ABC)
  • Positive, step 3
22
Q

What is Primary Effusion Lymphoma (PEL) associated with?

A

HHV8 and HIV

23
Q

DLBCL is what percentage of non-Hodgkin lymphomas?

A

30%

24
Q

DLBCL IHC:

-Positive for (5)

A
  • CD19
  • CD20
  • CD22
  • CD45
  • bcl-2
25
Q

DLBCL IHC:

-Variable for (3)

A
  • CD5
  • CD10
  • bcl-6
26
Q

What rearrangement is more common in GCB-type DLBCL?

A

BCL2

-t(14;18)

27
Q

Primary CNS DLBCL:

-Molecular/Cytogenetics

A

Most cases have BCL6 rearrangement
-Overexpress bcl-6

*BCL2 rearrangement is RARE

28
Q

Intravascular Large B cell lymphoma is AKA (3).

  • Symptoms due to what?
  • Nodal or Extranodal
A

-Angioednotheliomatosis
-Angiotropic lymphoma
-Intravascular lymphomatosis
Small vessel obstruction by large B cells
-LN involvement is RARE

29
Q

Where is the BCL6 gene located?

  • What is it commonly rearranged with?
  • Non-GCB (ABC) type or GCB type
A

3(q27)

  • t(3;14)
  • Non-GCB (ABC)
30
Q

What is HHV8/HIV associated with? (3)

A
  • PEL
  • Castleman
  • Kaposi
31
Q

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

-Large B cell IHC (+3;-3)

A 
Positive:
-pan B-cell
-bcl-6
-EMA (+/-)
Negative:
-CD15
-CD30
-EBV
32
Q

Where is the BCL2 gene located?

-Non-CGB (ABC) type or GCB type?

A

18(q21)

-GCB type

33
Q

Lymphomatoid Granulomatosis (LG) morphology and cell types.

A
  • Vasculitis pattern (Large Bs invade vessel wall)
  • Large number of reactive T-cells, plasma cells, and histiocytes
  • Granulomas UNcommon
34
Q

PTLD Risk Factors:

  • Renal/BMT recipients
  • Heart-Lung/Liver-Bowel
  • Age
  • Pretransplant EBV status
  • Degree of Immunosuppression
A

PTLD Risk Factors:

  • Renal/BMT- Lower Risk
  • Heart-Lung/Liver-Bowel - Higher Risk
  • Age - Kids at Higher Risk
  • Pretransplant EBV status - Negative
  • Degree of Immunosuppression - Most suppressed; Highest risk
35
Q

Plasmablastic DLBCL:

  • Neoplastic cells
  • Positive IHC (5)
  • Negative IHC (3)
  • Nodal or Extranodal
  • HIV or EBV association
A 
-Immunoblastic/Plasmablastic
Positive IHC:
-CD38
-CD138
-MUM1 (IRF4)
-cytoplasmic Ig
-EBV
Negative IHC: (contrast to plasmacytoma)
-CD20
-CD45
-CD56
Extranodal
-mucosa of oral cavity
Ass. w/ HIV and EBV
36
Q

What are the most common Lymphoproliferative disorders (LPD) associated with Primary Immunodeficiencies (PID)? (3)

A
  • DLBCL
  • Lymphomatoid Granulomatosis
  • T-Cell neoplasm
37
Q

Primary Effusion Lymphoma IHC:

-Positive for (6)

A
  • HHV8
  • CD30
  • CD45
  • CD38
  • CD138
  • EMA
38
Q

Primary CNS DLBCL:

  • Age
  • Radiographic
  • May present or recur as
  • Histology
A

Primary CNS DLBCL:

  • 60 y/0
  • Supratentorial mass (looks like GBM)
  • Intraocular lymphoma
  • Perivascular cuffs (express pan B cell antigens)

Boards.CP.Heme (15 decks)

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